History

Fact Explanation
Chronic productive cough Cough is the commonest symptom. It is caused by stimulation of the cough receptors in the air way by mucus. It is productive in nature in most cases. Color and amount of the sputum also should be noted. Usually it is purulent due to release of neutrophil myeloperoxidase. Sputum can give rise to an offensive odor. Sputum production may depend on body position of the patient according to the site of affected bronchi. [1,2,3]
Hemoptysis Erosive damage to the air way can give rise to blood stained sputum or frank hemorrhage.[1]
Non specific respiratory symptoms - dyspnea, wheeze, chest pain Progressively or intermittently can occur with infective exacerbations. Shortness of breath and wheeze occur due to air flow limitation.[2]
History of recurrent lower respiratory tract infections In bronchiectasis, inflamed, thickened bronchial walls are irreversibly damaged and natural defense mechanisms (mucociliary transport mechanism) are impaired. As a result of this mucus get accumulated and frequent bacterial infections can occur.[1,3,4]
Inadequate weight gain in children Bronchiectasis is a chronic disease with infective exacerbations which cause failure to thrive in children.[1,2]
History of immunodeficient state Immunodeficient state is a known etiology of bronchiectasis. Frequent respiratory tract infections due to impaired host response will damage the bronchi and cause bronchiectasis. [1,2,4,5]
History of foreign body aspiration Aspirated foreign bodies obstruct the bronchi and block the normal drainage of mucus providing good culture medium for the growth of micro-organisms leading to bronchiectasis. This is the commonest cause for bronchiectasis in children, particularly toddlers.[1,5]
History of childhood pneumonia, pertussis,measles, tuberculosis These are known etiology for bronchiectasis.[1,4]
Personal or family history of cystic fibrosis Cystic fibrosis is the most common cause of bronchiectasis in developed countries. It is an autosomal recessive inherited disorder so there can be a positive family history of cystic fibrosis.[1,2,4,5]
History of rheumatoid arthritis or inflammatory bowel disease These two disorders are associated with bronchiectasis.[1,2,5]
References
  1. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/
  2. American lung association.[online] [viewed 16.04.2014] Available from: http://www.lung.org/lung-disease/bronchiectasis/
  3. PAUL T KING. The pathophysiology of bronchiectasis. Internal Journal of Chronic Obstructive Pulmonary Disease.[online] 2009; 4: 411–419. [viewed 21.04.2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2793069/
  4. Bronchiectasis.[online] [viewed 21.04.2014] Available from: http://www.thoracic.org/education/breathing-in-america/resources/chapter-4-bronchiectasis.pdf
  5. Alan F. Barker. Bronchiectasis. New England Journal of Medicine.[online] 2002; 346:1383-1393. [viewed 21.04.2014] Available from: DOI: 10.1056/NEJMra012519

Examination

Fact Explanation
Early inspiratory coarse crackles on auscultation of the lungs Commonly heard in lower lung zones. In bronchiectasis there is abnormal, permanently dilated bronchi, usually with associated infection. [1,2]
Wheeze In bronchiectasis bronchi are filled with mucus limiting the airflow which give rise to wheeze.[1,2]
Digital clubbing An important clinical sign. But it is not specific for bronchiectasis. Also can occur in lung cancer, interstitial fibrosis, other suppurative lung conditions.[3]
Cyanosis Due to air flow limitation there is impaired gas exchange at alveolar level causing hypoxia. This in turn increase the unsaturated hemoglobin level in blood giving rise to bluish discoloration of peripheries.[1,2]
Signs of right heart failure - ankle oedema, tender hepatomegaly, elevated jugular venous pressure Severe cases of bronchiectasis can progressed into cor pulmonale causing right heart failure which will in turn produce these signs.[1,2]
References
  1. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/
  2. PAUL T KING. The pathophysiology of bronchiectasis. Internal Journal of Chronic Obstructive Pulmonary Disease.[online] 2009; 4: 411–419. [viewed 20.04.2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2793069/
  3. Malay Sarkar, D. M. Mahesh, and Irappa Madabhavi. Digital clubbing. Lung India : Official Organ of Indian Chest Society.[online] 2012 Oct-Dec;29(4): 354-362. [viewed 23.04.2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3519022/

Differential Diagnoses

Fact Explanation
Asthma Wheezing may be prominent with a diurnal pattern. Consider in patients with family history of asthma, atopy and history of environmental risk factors such as allergic exposure, occupational sensitizers.[1,6]
Chronic obstructive pulmonary disease (COPD) Smoking is a well known risk factor and usually patients give a history of smoking. Suspect if there is shortness of breath and effort limitations due to the fixed airflow obstruction which is characteristic in COPD.[2,6]
Gastro-esophageal reflux disease (GORD) GORD causes aspiration of food particles due to regurgitation. So these patients often developed aspiration associated irritation of the throat and upper airway which causes chronic cough. Consider in patients with other dyspeptic symptoms.[3]
Drug induced cough Angiotensin Converting Enzyme inhibitors are well known drug to cause dry cough which is troublesome. Commonly affect women than men. Check patients drug history carefully to exclude this. [3]
Idiopathic pulmonary fibrosis Cigarette smoking, variety of environmental exposures such as metal dust, wood dust, animal dust, microbial agents play a role as risk factors for the development of idiopathic pulmonary fibrosis. On examination late inspiratory fine crackles will be heard in pulmonary fibrosis.[7]
Tuberculosis Patients will present with fever, night sweats, productive cough, hemoptysis, loss appetite, weight loss. Consider if there is a contact history with a known patient with tuberculosis, travel history to an endemic area, past personal history of tuberculosis. Examination will reveal enlarged lymph nodes, pleural effusions which favor tuberculosis. [5]
Lung cancer Early stages give no symptoms and signs, so difficult to diagnose. Consider in heavy smokers as it is the main risk factor for lung cancer. Non specific systemic symptoms, hemoptysis will be prominent and minority will present with paraneoplastic syndrome. [4]
References
  1. SUSAN M. POLLART, KURTIS S. ELWARD. Overview of Changes to Asthma Guidelines: Diagnosis and Screening. Am Fam Physician.[online] 2009 May 1;79(9):761-767. [viewed 21.04.2014] Available from: http://www.aafp.org/afp/2009/0501/p761.html
  2. MARVIN DEWAR, R. WHIT CURRY. Chronic Obstructive Pulmonary Disease: Diagnostic Considerations. Am Fam Physician.[online] 2006 Feb 15;73(4):669-676. [viewed 21.04.2014] Available from: http://www.aafp.org/afp/2006/0215/p669.html
  3. ROBERT L. HOLMES, CLARE T. FADDEN. Evaluation of the Patient with Chronic Cough. Am Fam Physician.[online] 2004 May 1;69(9):2159-2166. [viewed 21.04.2014] Available from: http://www.aafp.org/afp/2004/0501/p2159.html
  4. LAUREN G. COLLINS, CHRISTOPHER HAINES, ROBERT PERKEL, ROBERT E. ENCK. Lung Cancer: Diagnosis and Management. Am Fam Physician.[online] 2007 Jan 1;75(1):56-63. [viewed 21.04.2014] Available from: http://www.aafp.org/afp/2007/0101/p56.html
  5. ANTHONY F. JERANT, MICHELLE BANNON, STEPHEN RITTENHOUSE. Identification and Management of Tuberculosis. Am Fam Physician.[online] 2000 May 1;61(9):2667-2678. [viewed 21.04.2014] Available from: http://www.aafp.org/afp/2000/0501/p2667.html
  6. Rodrigo Athanazio. Airway disease: similarities and differences between asthma, COPD and bronchiectasis. Clinics (Sao Paulo). [online] Nov 2012; 67(11): 1335–1343. [viewed 21.04.2014] Available from: doi: 10.6061/clinics/2012(11)19 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3488995/
  7. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. American thoracic society documents.[online] [viewed 22.04.2014] Available from: http://www.thoracic.org/statements/resources/interstitial-lung-disease/ipf0311.pdf

Investigations - for Diagnosis

Fact Explanation
Chest X-ray Baseline chest X-ray should be done in all patients. The main role is to exclude other differential diagnoses.[1,2]
High-resolution computed tomography (HRCT) Investigation of choice to confirm the diagnosis of bronchiectasis. It will show that internal diameter of an affected bronchus is wider than its adjacent pulmonary artery, absence of tapering of bronchi peripherally and appearance of bronchi in the outer 1–2 cm of the lung fields which are diagnostic of bronchiectasis.[1,3]
Sweat chloride or gene testing (CFTR genetic mutation analysis) To diagnose cystic fibrosis, for all children and adults up to 40 years of age and adults older than 40 years of age with clinical features consistent with cystic fibrosis.[1]
Serum IgG (immunoglobulin G), IgA, IgM and electrophoresis As a screening for gross antibody deficiency, for all people with confirmed bronchiectasis.[1]
Alpha-1 antitrypsin level, serum IgE, Aspergillus-specific IgE and precipitins For people with clinical features or risk factors for immunological disorders to find out the underlying cause for bronchiectasis.[1,2]
Bronchoscopy with mucosal biopsy Indicated when suspecting a bronchial obstruction (eg: in single lobe bronchiectasis, suspected foreign body aspiration in children or to exclude an endobronchial lesion in adults).[1,2,3]
Gastrointestinal investigations - 24-hour pH monitoring For patients suspected of having gastro-esophageal reflux disease.[1,2]
Sputum - gram stain, culture and sensitivity testing To diagnose the causative micro-organism and decide the antibiotic treatment.[1,2]
Pulmonary function Spirometry will reveal air flow limitation with a reduced ratio of forced expiratory volume in one second (FEV1) to forced vital capacity (FVC), a normal or slightly reduced FVC, and a reduced FEV1. Severity of bronchiectasis can be assessed using lung function tests. Reversibility testing may identify improvement in lung function after bronchodilators.[1,2]
Full blood count and white cell differential count Changes can be seen during an infective exacerbation. Also patient's hemoglobin level can be assessed.[1]
Erythrocyte sedimentation rate (ESR) or C reactive protein (CRP) These are indirect markers of disease activity and elevated levels can be found in an infective exacerbation.[1]
References
  1. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/
  2. Alan F. Barker. Bronchiectasis. New England Journal of Medicine.[online] 2002; 346:1383-1393. [viewed 21.04.2014] Available from: DOI: 10.1056/NEJMra012519 http://www.nejm.org/doi/full/10.1056/NEJMra012519
  3. PAUL T KING. The pathophysiology of bronchiectasis. Internal Journal of Chronic Obstructive Pulmonary Disease.[online] 2009; 4: 411–419. [viewed 20.04.2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2793069/

Investigations - Followup

Fact Explanation
Sputum for culture and sensitivity Should be done annually to assess for chronic bacterial colonization.[1,2]
Pulmonary function tests Spirometry should be done at least annually to assess the lung function with the pulmonary rehabilitation program.[1,2]
High resolution computed tomography (HRCT) HRCT images should be reviewed to assess the success of the physiotherapy techniques and also to plan suitable, individualized clearance techniques.[1,2]
References
  1. C. Elaine Field. Bronchiectasis.A Long-term Follow-up of Medical and Surgical Cases from Childhood. Arch Dis Child. [online] Dec 1961; 36(190): 587–603. [viewed 20.04.2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2012811/
  2. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/

Management - General Measures

Fact Explanation
Patient and family education Should educate about the chronic nature of the disease. Also should mention that it is not curable but quality of life can be improved by proper treatment.[1,2]
Immunization Offer immunization against Streptococcus pneumoniae and seasonal influenza. Also effective childhood immunization program against pertussis, measles will prevent the occurrence of bronchiectasis as a result of childhood infections.[1,2]
Diet Since this is a chronic disease adequate nutritional intake with supplementation is necessary. So patients' diet should be assessed closely and should make necessary changes, specially in children.[2]
Help stopping smoking Smoking also impair the mucociliary defense mechanism and cause accumulation of mucus which can worsen bronchiectasis. Therefore help stopping smoking for those who smoke and also avoid passive smoking in all patients.[2]
References
  1. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/
  2. Alan F. Barker. Bronchiectasis. New England Journal of Medicine.[online] 2002; 346:1383-1393. [viewed 21.04.2014] Available from: DOI: 10.1056/NEJMra012519 http://www.nejm.org/doi/full/10.1056/NEJMra012519

Management - Specific Treatments

Fact Explanation
Antibiotic therapy - for infective exacerbation Empirical antibiotics should be started once the diagnosis of acute infective exacerbation is made clinically. First-line treatment is amoxicillin 500 mg three times a day or clarithromycin 500 mg twice daily (in patients that are penicillin-allergic) for 14 days. Higher dose may be needed in patients with severe bronchiectasis chronically colonized with H influenzae. Ciprofloxacin should be used in patients colonized with Pseudomonas aeruginosa. Intravenous antibiotics should be given for unwell patients, who have resistant organisms or have failed to respond to oral therapy. Note: antibiotics should be changed according to the culture results and sensitivity pattern.[1]
Antibiotic therapy - prophylactically More than 3 exacerbations per year requiring antibiotic therapy or fewer severe exacerbations indicate the need of long-term antibiotics. Long-term nebulized antibiotics can be used, specially for children.[1]
Bronchodilators Dilatation of the airway will areate the lung sufficiently and reduce shortness of breath and facilitate mucus drainage. First have to assess patients symptom improvement with beta 2 agonist and anticholinergic bronchodilators and should prescribe therapy where lung function or symptoms improve on bronchodilators.[1,2]
Mucolytics and humidification To clear the air way which in turn improve the air flow. Sterile water inhalation, normal saline nebulization will facilitate mucus clearance.[1,2]
Leukotriene receptor antagonists. Not recommended for the treatment of bronchiectasis.[1]
Corticosteroids - inhaled or oral Not recommended unless there is co-existent asthma.[1]
Physiotherapy Airway clearance techniques are important in managing bronchiectasis as it is necessary to get rid of the mucus to break the vicious cycle and also to improve quality of the life by reducing the number of exacerbations. Techniques such as postural drainage, forced expiration, active cycle of breathing should be included.[1,2]
Pulmonary Rehabilitation Recommended for patients who got limited exercise capacity/breathlessness with activities of daily living. Inspiratory muscle training will improve exercise endurance.[1,2]
Surgery Consider lung resection surgery in people with localized disease when symptoms cannot be controlled by medical treatment. Consider lung transplantation in patients with cystic fibrosis and respiratory failure.[1,2]
Non invasive ventilation (NIV) NIV can improve quality of life in some patients with chronic respiratory failure due to bronchiectasis.[1,2]
Hemoptysis management Bronchial artery embolisation and/or surgery is treatment of choice for the management of massive hemoptysis.[1,2]
Making refferals Patients should be referred to a chest physician, physiotherapist and respiratory nurse for further necessary management.[1,2]
References
  1. M C PASTEUR, D Bilton, A T Hill. British Thoracic Society guideline for non-CF bronchiectasis. Thorax; an international journal of respiratory medicine.[online] July 2010. vol 65, supplement 1. [viewed 16.04.2014] Available from: https://www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/
  2. Alan F. Barker. Bronchiectasis. New England Journal of Medicine.[online] 2002; 346:1383-1393. [viewed 21.04.2014] Available from: DOI: 10.1056/NEJMra012519 http://www.nejm.org/doi/full/10.1056/NEJMra012519